Clinical and gene mutation characteristics of patients with hereditary ellipsocytosis: nine cases report and literature review / 中华血液学杂志

Objective: To report gene mutations in nine patients with hereditary elliptocytosis (HE) and analyze the characteristics of pathogenic gene mutations in HE. Methods: The clinical and gene mutations of nine patients clinically diagnosed with HE at Institute of Hematology & Blood Diseases Hospital from June 2018 to February 2022 were reported and verified by next-generation sequencing to analyze the relationship between gene mutations and clinical phenotypes. Results: Erythrocyte membrane protein gene mutations were detected among nine patients with HE, including six with SPTA1 mutation, one with SPTB mutation, one with EPB41 mutation, and one with chromosome 20 copy deletion. A total of 11 gene mutation sites were involved, including 6 known mutations and 5 novel mutations. The five novel mutations included SPTA1: c.1247A>C (p. K416T) in exon 9, c.1891delG (p. A631fs*17) in exon 15, E6-E12 Del; SPTB: c.154C>T (p. R52W) ; and EPB41: c.1636A>G (p. I546V) . Three of the six patients with the SPTA1 mutation were SPTA1 exon 9 mutation. Conclusion: SPTA1 is the most common mutant gene in patients with HE.

Advances in the classification and treatment of isolated superior mesenteric artery dissection / 中华外科杂志

Isolated superior mesenteric artery dissection (ISMAD) has attracted more and more clinicians' attention in recent years. Patients onset of ISMAD often present with abdominal pain. The misdiagnosis or miss diagnosis is common because of the non-specific symptoms and signs, which even can endanger lives in serious cases. Imaging classification is of great significance for diagnosis and treatment of ISMAD. The Sakamoto classification and the Yun classification are two classical classified methods. However, with the further study of ISMAD, various new classifications emerge. Conservative treatment was once considered as the preferred. As the rapid development of endovascular therapy and the great progress of new devices, stenting therapy can significantly improve symptoms and achieve satisfactory long-term effects, and be even expected to become the preferred method for clinical therapy of ISMAD. However, the long-term effects of endovascular therapy still need a large number of follow-up data, and complications after stent implantation can't be ignored.

Characteristics of bone marrow compensatory erythropoiesis in hereditary spherocytosis / 中华血液学杂志

Objective: To reveal the compensatory features of bone marrow (BM) erythropoiesis in hereditary spherocytosis (HS) and to explore the effect of diferent hemoglobin levels on this compensation. Methods: Clinical and laboratory data of patients with HS were collected, and the peripheral blood absolute reticulocytes counts value was taken as the surrogate parameter to evaluate the ability of erythropoiesis compensation. BM erythropoiesis compensation in HS with diferent degrees of anemia were evaluated. Results: ①Three hundred and two patients were enrolled, including 115 with compensated hemolytic disease, 74 with mild anemia, 90 with moderate anemia, and 23 with severe anemia. ②Hemoglobin (HGB) was negatively correlated with serum erythropoietin in the decompensated hemolytic anemia group (EPO; rs=-0.585, P<0.001) . ③The median absolute reticulocyte count (ARC) of HS patients was 0.34 (0.27, 0.44) ×10(12)/L, up to 4.25 times that of normal people. The maximum ARC was 0.81×10(12)/L, about 10 times that of normal people. The median ARC of patients with compensated hemolytic disease was 0.29 (0.22, 0.38) ×10(12)/L, up to 3.63 times that of normal people. The median ARC of patients with hemolytic anemia was 0.38 (0.30, 0.46) ×10(12)/L, which was significantly higher than the patients with compensated hemolytic disease, up to 4.75 times that of normal people (z=4.999, P=0.003) . ④ ARC was negatively correlated with HGB in the compensated hemolytic disease group (rs=-0.177, P=0.002) and positively correlated with HGB in the decompensated hemolytic anemia group (rs=0.191, P=0.009) . There was no significant difference in the ARC among patients with mild, moderate, and severe anemia (χ(2)=4.588, P=0.101) . ⑤The median immature reticulocyte production index of the mild, moderate, and severe anemia groups was 13.1% (9.1%, 18.4%) , 17.0% (13.4%, 20.8%) , and 17.8% (14.6%, 21.8%) , respectively; the mild anemia group had lower index values than the moderate and severe anemia groups (P(adj) values were both<0.05) , but there was no significant difference between the latter groups (P(adj)=1.000) . The median immature reticulocyte count of patients in the mild, moderate, and severe groups was 5.09 (2.60, 7.74) ×10(10)/L, 6.24 (4.34, 8.83) ×10(10)/L, and 7.00 (3.07, 8.22) ×10(10)/L, respectively; there was no significant difference among the groups (χ(2)=3.081, P=0.214) . Conclusion: HGB can be maintained at a normal level through bone marrow erythropoiesis, while red blood cells are reduced in HS. However, once anemia develops, the bone marrow exerts its maximum erythropoiesis capacity and does not increase, regardless of anemia aggravation or serum EPO increase.

Effect of Shenling Baizhusan on Electrogastrogram in Children with Spleen Deficiency Diarrhea / 中国实验方剂学杂志

ObjectiveTo study the effect of Shenling Baizhusan on electrogastrogram in children with spleen deficiency diarrhea. To clarify the occurrence of gastric electrical rhythm disorder in children with this disease， and to study whether Shenling Baizhusan can improve the abnormal gastric motility in children with diarrhea （spleen deficiency） MethodA total of 125 children with spleen deficiency diarrhea in the outpatient department of Children's Hospital of Shanghai from October 2019 to March 2021 were selected as the research objects， and they were randomly divided into a control group （60 cases） and an observation group （65 cases）. The children in the control group were treated with Montmorillonite powder combined with probiotics treatment， and the children in the observation group were additionally treated with Shenling Baizhusan. The course of treatment for both groups was 1 week. The clinical efficacy of the two groups of children after treatment and the scores of main traditional Chinese medcine（TCM） symptoms before and after treatment were compared， and the changes in the main parameters of electrogastrogram in children before and after treatment were compared. ResultAfter treatment， the total effective rate of observation group （90.77%， 59/65） was higher than that of control group （76.67%，46/60） （χ2=4.617， P<0.05）. After treatment， scores of fecal morphology， frequency of defecation， fatigue， inappetence， and other symptoms in both groups were lower than that before treatment （P<0.05）， and the observation group was lower than the control group （P<0.05）. As compared with before treatment， the main frequency， the percentage of normal slow wave， and the percentage of normal gastric electrical rhythm in the two groups increased after treatment （P<0.05）， and the control group was lower than the observation group （P<0.05）. The proportion of children with slow gastric rhythm decreased （P<0.05） as compared with before treatment， and the control group was higher than the observation group （P<0.05）. ConclusionShenling Baizhusan can significantly relieve the diarrhea symptoms in children with spleen deficiency diarrhea and improve gastric motility with good clinical effects.

Clinical Randomized Controlled Observation of Modified Wuhutang in Treatment of Acute Asthma in Children / 中国实验方剂学杂志

ObjectiveTo explore the clinical effect of modified Wuhutang in the treatment of children with acute asthma. MethodA total of 130 children with acute asthma were randomly divided into an observation group and a control group， with 65 cases in each group. The observation group was treated with modified Wuhutang and the control group was treated with procaterol hydrochloride for one week. The scores of primary symptoms （wheezing， cough， shortness of breath， and chest tightness） and secondary symptoms （mental status， runny nose， dry mouth， tongue texture， tongue coating， stool， etc.）， lung functions， immunoglobulin E （IgE） expression， eosinophil （EOS） count， and serum inflammatory factors， including interleukin （IL）-5， IL-6， IL-8， and IL-1β in two groups before and after treatment were compared. ResultThe data of 126 children were statistically analyzed. As revealed by the results， compared with the conditions before treatment， the scores of primary symptoms and secondary symptoms， serum levels of IL， IgE expression， and EOS count were both reduced in two groups （P<0.05）， lung functions were increased in the two groups（P<0.05）. Compared with the control group after treatment， the observation group showed decreased scores of cough and secondary symptoms （P<0.05）， and insignificant decrease in IL-1β （P<0.05）. The improvement in lung functions， IgE expression， and EOS count in observation group was equivalent to that in control group. ConclusionModified Wuhutang for treatment of acute asthma in children （phlegm-heat obstructing lung syndrome） can significantly relieve the clinical symptoms， improve lung functions， and reduce IgE， IL-5， IL-6， IL-8， IL-1β expression levels and EOS count， and its overall clinical efficacy is superior or equivalent to that of tprocaterol hydrochloride.

Pharmacokinetic study of anti-human T-cell porcine immunoglobulin combined with cyclosporine A immunosuppressive therapy in patients with severe aplastic anemia / 中华血液学杂志

Objective: To study the metabolic characteristics of anti-human T-cell porcine immunoglobulin (p-ATG) in patients with severe aplastic anemia (SAA) . Methods: For patients with SAA treated with p-ATG combined cyclosporine A (CsA) immunosuppressants between February 2017 and December 2017, the p-ATG dose was 20 mg·kg(-1)·d(-1) over 12 h of intravenous administration for 5 consecutive days. The blood concentration of p-ATG was detected by the three-antibody sandwich ELISA method, the pharmacokinetic analysis software was fitted, and the second-chamber model method was used to calculate the pharmacokinetic parameters and plot the pharmacokinetic curve. Adverse events were recorded and the hematologic reactions were determined at 6 months after treatment. Results: Sixteen patients with SAA treated with p-ATG were enrolled, including 8 females and 8 males, with a median age of 22 years (range, 12 to 49 years) and a median weight of 62.5 kg (range, 37.5 to 82.0 kg) . The pharmacokinetics of p-ATG could be evaluated in 14 cases. p-ATG is distributed in vivo as a two-chamber model, with an average drug concentration peak (T(max)) of (5.786±2.486) days, a peak concentration (C(max)) of (616±452) mg/L, and a half-life of (10.479±8.242) days. The area under the drug time curve (AUC) was (5.807±3.236) mg/L·d. Six months after treatment, 8 of 14 patients received a hematologic response; the AUC (0-t) of the effective group and ineffective groups was (7.50±3.26) mg/L·d vs (4.50±2.18) mg/L·d, and the C(max) was (627±476) mg/L vs (584±382) mg/L, respectively. Conclusion: The plasma concentration of p-ATG reached a peak after 5 days of continuous infusion, and then decreased slowly, with a half-life of 10.479 days, and the residual drug concentration was detected in the body 60 days after administration. A relationship between drug metabolism and efficacy and adverse reactions could not be determined.

Reassessing the six months prognosis of patients with severe or very severe aplastic anemia without hematological responses at three months after immunosuppressive therapy / 中华血液学杂志

Objective: To reassess the predictors for response at 6 months in patients with severe or very severe aplastic anemia (SAA/VSAA) who failed to respond to immunosuppressive therapy (IST) at 3 months. Methods: We retrospectively analyzed the clinical data of 173 patients with SAA/VSAA from 2017 to 2018 who received IST and were classified as nonresponders at 3 months. Univariate and multivariate logistic regression analysis were used to evaluate factors that could predict the response at 6 months. Results: Univariate analysis showed that the 3-month hemoglobin (HGB) level (P=0.017) , platelet (PLT) level (P=0.005) , absolute reticulocyte count (ARC) (P<0.001) , trough cyclosporine concentration (CsA-C0) (P=0.042) , soluble transferrin receptor (sTfR) level (P=0.003) , improved value of reticulocyte count (ARC(△)) (P<0.001) , and improved value of soluble transferrin receptor (sTfR(△)) level (P<0.001) were related to the 6-month response. The results of the multivariate analysis showed that the PLT level (P=0.020) and ARC(△) (P<0.001) were independent prognostic factors for response at 6 months. If the ARC(△) was less than 6.9×10(9)/L, the 6-month hematological response rate was low, regardless of the patient's PLT count. Survival analysis showed that both the 3-year overall survival (OS) [ (80.1±3.9) % vs (97.6±2.6) %, P=0.002] and 3-year event-free survival (EFS) [ (31.4±4.5) % vs (86.5±5.3) %, P<0.001] of the nonresponders at 6 months were significantly lower than those of the response group. Conclusion: Residual hematopoietic indicators at 3 months after IST are prognostic parameters. The improved value of the reticulocyte count could reflect whether the bone marrow hematopoiesis is recovering and the degree of recovery. A second treatment could be performed sooner for patients with a very low ARC(△).

Relationship between Changes of Lymphocyte Subsets and Early Hematologic Response in Immunosuppressive Therapy of Severe Aplastic Anemia Patients / 中国实验血液学杂志

OBJECTIVE@#To explore the relationship between the change of lymphocyte subsets before and after immunosuppressive therapy (IST) with disease severity of severe aplastic anemia (SAA) and hematologic response to IST.@*METHODS@#The clinical data of 94 patients with SAA/VSAA treated by r-ATG and CsA in our hospital from December 2009 to October 2011 was analyzed retrospectively. Among them, 26 patients who had sequential data of lymphocyte subsets and cytokines before and after treatment were enrolled. The relationship between lymphocyte subsets, cytokine level before IST and disease severity, as well as the relationship between changes if lymphocyte subsets, changes of cytokine and the HR after IST for 6 months was analyzed.@*RESULTS@#There were no statistical differences in the ratio and absolute count of lymphocyte, the ratio and absolute count of each lymphocyte subsets, including CD3@*CONCLUSION@#The hematopoietic recovery and early hematologic remission may be affected by the intensity of immune suppression reflected from the changes of lymphocyte subsets and the immune reconstruction reflected from the recovery of lymphocyte subsets. The immune reconstruction is most significant within 3 months after IST.

Effects of Human Immunodeficiency Virus-positive Mothers Receiving Antiretroviral Therapy to Prevent Mother-to-child Transmission on the Growth and Development of 18-month-old Children in Lingshan County of Guangxi / 中国医学科学院学报

Objective To evaluate the effects of antiretroviral therapy(ART)for the prevention of mother-to-child transmission(PMTCT)of acquired immune deficiency syndrome(AIDS)on the growth and development of 18-month-old children born by human immunodeficiency virus(HIV)-positive pregnant women in Lingshan County,Guangxi Zhuang Autonomous Region,and provide scientific evidence for improving the ART medication plan for PMTCT.Methods Lingshan County,ranking the first in the HIV-epidemic counties of Guangxi,was selected as the research site.According to the design of retrospective case-control study,we assigned all the subjects into the case group and the control group:(1)The case group included the HIV-positive pregnant women who had received ART for PMTCT and their HIV-negative infants in Lingshan County from 2010 to 2017.The historical cards and PMTCT data of them were collected from the national PMTCT database.(2)The control group included the healthy pregnant women and their healthy babies born in the Lingshan Maternity and Infant Hospital in 2017,and the children's growth and development data were collected.The stunted growth in children was defined as at least one of the three main indicators of body height,body weight,and head circumference below the normal range.Results The number of HIV-positive mothers and their infants in the case group was 391 and 368,respectively,and 87.21%(341/391)and 95.38%(351/368)of mothers and infants respectively received ART medication.The HIV positive rate,mortality rate,and mother-to-child transmission rate of 18-month-old children were 1.36%(5/368),4.35%(16/368),and 2.01%(5/249),respectively.The incidence of stunted growth of 18-month-old children in the case group and the control group was 42.12%(155/368)and 23.06%(101/438),respectively,with significant difference(

Blockade of HCN2 Channels Provides Neuroprotection Against Ischemic Injury via Accelerating Autophagic Degradation in Hippocampal Neurons / 神经科学通报·英文版

In the central nervous system, hyperpolarization-activated cyclic nucleotide-gated (HCN) channels are essential to maintain normal neuronal function. Recent studies have shown that HCN channels may be involved in the pathological process of ischemic brain injury, but the mechanisms remain unclear. Autophagy is activated in cerebral ischemia, but its role in cell death/survival remains controversial. In this study, our results showed that the HCN channel blocker ZD7288 remarkably decreased the percentage of apoptotic neurons and corrected the excessive autophagy induced by oxygen-glucose deprivation followed by reperfusion (OGD/R) in hippocampal HT22 neurons. Furthermore, in the OGD/R group, p-mTOR, p-ULK1 (Ser), and p62 were significantly decreased, while p-ULK1 (Ser), atg5, and beclin1 were remarkably increased. ZD7288 did not change the expression of p-ULK1 (Ser), ULK1 (Ser), p62, Beclin1, and atg5, which are involved in regulating autophagosome formation. Besides, we found that OGD/R induced a significant increase in Cathepsin D expression, but not LAMP-1. Treatment with ZD7288 at 10 μmol/L in the OGD/R group did not change the expression of cathepsin D and LAMP-1. However, chloroquine (CQ), which decreases autophagosome-lysosome fusion, eliminated the correction of excessive autophagy and neuroprotection by ZD7288. Besides, shRNA knockdown of HCN2 channels significantly reduced the accumulation of LC3-II and increased neuron survival in the OGD/R and transient global cerebral ischemia (TGCI) models, and CQ also eliminated the effects of HCN2-shRNA. Furthermore, we found that the percentage of LC3-positive puncta that co-localized with LAMP-1-positive lysosomes decreased in Con-shRNA-transfected HT22 neurons exposed to OGD/R or CQ. In HCN2-shRNA-transfected HT22 neurons, the percentage of LC3-positive puncta that co-localized with LAMP-1-positive lysosomes increased under OGD/R; however, the percentage was significantly decreased by the addition of CQ to HCN2-shRNA-transfected HT22 neurons. The present results demonstrated that blockade of HCN2 channels provides neuroprotection against OGD/R and TGCI by accelerating autophagic degradation attributable to the promotion of autophagosome and lysosome fusion.

Robot-assistedlaparoscopic ureteral reimplantation:A report of 5 cases / 医学研究生学报

Objective Reports are relatively few at home and abroad onrobot-assistedlaparoscopic ureteral reimplantation ( RAUR) .This study summarizes our experience with RAUR and assesses the feasibility and clinical effect of the strategy . Methods We retrospectively analyzed the clinical data about 5 cases of lower ureterostenosistreated by RAUR in our department from May 2015 to February 2017. Results Operations were successfully completed in all the cases , with an estimatedintraoperative blood loss of 40-100 mL but no blood transfusion , nor conversion to open surgery , nor intestinal or major vascular injury .The urethral catheter was re-moved at 5-7 days after surgery and there were no postoperative complications .The patients were followed up for over 6 months , during which all showed increased glomerular filtration rate ( 2-12 mL/min) of the involved kidney at 3 months and ultrasonography revealed no ureterostenosisorhydronephrosis . Conclusion Robot-assistedlaparoscopic ureteral reimplantation is a safe , effective and minimally invasive surgical option for distal ureteral obstruction .

Aggravated airway inflammation and resistance in a respiratory syncytial virus infected murine asthma model / 第二军医大学学报

Objective To explore the role of…respiratory, syncytial virus (RSV) infection in aggravating airway inflammation and airway resistance in murine-asthma model. Methods A total of 30 6-8-week-old male BALB/c mice were equally divided into three groups randomly: thecontrol group,’ ovalbumin (OVA) group (asthma group), and respiratory syncytial virus (RSV)/OVA group (RSVh asthma group). In OVA group,murine asthma model was established using an OVA sensitization; in OSV/RSV group,mice were firstly sensitized by OVA and subsequently infected with RSV intranasally for three times to make acute viral infection asthma model ; and in control group, the mice received equal volume of PBS treatment. Twenty-four hours after the last challenge,the airway resistance was evaluated by mouse ventilator (Buxco RC). Inflammatory cell infiltration was measured in bronchoalveolar lavage fluid (BALF). Pulmonary tissue samples were collected and stained with H E,PAS or VG to observe inflammation of pulmonary tissues. Results Pulmonary tissue of mice in OVA group had inflammatory cell infiltration, airway mucus secretions were visible, and collagen was seen around the airway. Pulmonary tissue of mice in OVA/RSV group had significant inflammatory cell infiltration, alveolar stenosis, telangiectasia congestion,airway mucus secretions and collagen deposition. Lung function and the proportion of BALF eosinophils (EOS%) in OVA group and OVA/RSV group were significantly different from that of the control group (P<0. 05,7<0. 01). Airway resistance and the EOS% in OVA/RSV group were significantly different from those of the OVA group (P<0. 05). Conclusion RSV infection can aggravate the airway inflammation and result in airway resistance in murine asthma model.

Multilayer stents, a new progress in the endovascular treatment of aneurysms / 中华医学杂志(英文版)

<p><b>OBJECTIVE</b>To review the recent progress of multilayer stents in treating arterial aneurysms and to draw an initial conclusion about its paradigm.</p><p><b>DATA SOURCES</b>PubMed database and ELSEVIER database were searched with the keywords "cardiatis" or "multilayer stent" for relevant articles from January 2008 to September 2012. Relevant websites (provided by Cardiatis) were also involved in the review process.</p><p><b>STUDY SELECTION</b>Well-controlled, relatively large-scale, retrospective studies as well as meaningful individual cases were all selected as materials.</p><p><b>RESULTS</b>A total of 23 articles were involved in this review. The newly introduced Cardiatis multilayer stent aims at creating an active flow-modulating barrier between normal blood flow and aneurismal sac, which can induce thrombosis within aneurismal sac and preserve collateral circulation at the same time. Currently, it has been applied for complicated aneurysms located in different segments of the arterial system.</p><p><b>CONCLUSION</b>This new concept of multilayer uncovered stent offers a promising alterative in the treatment of arterial aneurysms. However, a further large-scale clinical and hemodynamic study is required to evaluate the long-term effects.</p>

A novel pressure difference-induced perforation aortic stent-grafts system: an experimental study / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Most of endovascular stent-graft modifications to preserve side branch must be customized according to extensive pre-operative assessment, which may not be possible in many hospitals and emergency settings. The study was to develop a novel stent-grafts system that would allow in situ "fenestration", with less reliance on preoperative imaging.</p><p><b>METHODS</b>The magnitude of pressure difference (PD) between left subclavian artery (LSA) and aortic arch were measured in 12 experimental pigs. Changes of PD before and after LSA was covered were analyzed respectively. The novel stent graft was made by multi-dimensional and multiple textiles forming technology. According to the PD measurement in pigs, we evaluated the feasibility of the stent-graft in a mock circulation system.</p><p><b>RESULTS</b>In pigs, the blood pressure of aortic arch was significantly higher than that of LSA after it was covered (P < 0.001) and PD was (42.78 ± 5.17) mmHg. After target vessel was covered and when PD between the LSA and aorta reached the magnitude measured in pigs, contrast media oozed from the cranny of graft to the LSA, which was generated by sliding and deformation of yarns of novel stent-graft.</p><p><b>CONCLUSIONS</b>The study proposes the design of pressure difference-induced perforation aortic stent-grafts system and verifies that the PD between LSA and aortic arch is high enough to allow in situ "fenestration" by stent graft made by multi-dimensional and multiple textiles forming technology.</p>

Effect of diabetes on short-term prognosis of transient ischemic attack in elderly patients / 中华老年医学杂志

Objective To investigate the effect of diabetes on short-term prognosis of transient ischemic attack (TIA) in elderly patients.Methods From January 2006 to June 2010,126 patients with TIA aged over 60 years were selected.Patients were divided into diabetic group and non-diabetic group according to past history,blood glucose and glycosylated hemoglobin levels.The cumulative ischemic stroke incidences were analyzed by Kaplan-Meier survival analysis 30 days and 90 days after the first TIA.The risk factors for short-term stroke after TIA were analyzed by Cox regression analysis.Results Among 126 patients with TIA,31 cases (24.6％) had diabetes.The cumulative ischemic stroke incidences were significantly higher in diabetic group than in non-diabetic group 30 days and 90 days after the first TIA (54.8％ vs.22.1％,61.3％ vs.28.4％,both P＜0.01).Cox regression analysis revealed that diabetes and cerebral arterial stenosis were the risk factors for recurrent stroke within 90 days.Conclusions The short-term stroke incidence is significantly higher in elderly diabetic patients than in non-diabetic patients.Diabetes is the independent risk factor for recurrent stroke after TIA.

Predicting early response to immunosuppressive therapy in severe aplastic anemia by soluble transferrin receptor assay / 中华血液学杂志

<p><b>OBJECTIVE</b>To evaluate the value of serum soluble transferrin receptor (sTfR) concentration in predicting early response to immunosuppressive therapy (IST) in severe aplastic anemia (SAA).</p><p><b>METHODS</b>Clinical data and hematologic responses of 140 SAA patients treated with rabbit antithymocyte globulin (rATG) combination with cyclosporine in our hospital were retrospectively analyzed. Correlation of pre-IST baseline of sTfR and IST responses was statistically analyzed and receiver operating characteristic (ROC) curve was used to estimate the sensitivity and specificity of sTfR in prediction of early responses.</p><p><b>RESULTS</b>Serum concentration of sTfR in very SAA (VSAA) patients were significantly lower than SAA and transfusion dependent non-SAA cases (P=0.001). The responders, especially at 3 months, had significantly higher pre- IST baseline of sTfR [median, 0.89 (range, 0.21-2.42) mg/L] than that [median, 0.58 (range, 0.13-1.88) mg/L] of non-responders (P=0.005). The cutoff level of 0.91 mg/L and 0.88 mg/L for predicting responses at 3 and 6 months were established based on the ROC curve, with the degree of accuracy of 65.0% and 60.7% respectively. Multivariate analysis showed that pre-IST baseline of sTfR was the independent factor of predicting response at 3 months (P=0.007) and at 6 months (P=0.021).</p><p><b>CONCLUSION</b>As a indicator of bone marrow failure severity, sTfR could predict early response to IST therapy in aplastic anemia.</p>

High-dose cyclophosphamide for severe aplastic anemia associated with β-thalassemia: a case report and literatures review / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical features and therapeutic method for severe aplastic anemia (SAA) associated with β-thalassemia, and to improve the recognition of the disease.</p><p><b>METHODS</b>One patient hospitalized for pancytopenia was reported and the related literatures were reviewed.</p><p><b>RESULTS</b>A 14-years old girl who presented with anemia from her childhood was hospitalized for acute onset of pancytopenia. Routine blood test showed that WBC count was 1.28×10⁹/L, hemoglobin 65 g/L, platelet count 18×10⁹/L, reticulocyte count 2×10⁹/L, neutrophil count 0.03×10⁹/L and mean corpuscular volume 59.6 fl, respectively. Both bone marrow aspiration and biopsy showed hypoplasia. Her red blood cells presented as microcytic hypochromic and target erythrocytes were common on peripheral blood smear. DNA analysis of the patient and her mother showed exon 17 heterozygous β-thalassemia (c.52 A>T). A diagnosis of SAA associated with β-thalassemia was clarified and high-dose cyclophosphamide (HD-CTX, 1.2 g/d×4 d) plus cyclosporine were offeved, which eventually led to a complete hematologic remission 12 months later.</p><p><b>CONCLUSION</b>This was the first report of SAA associated with β-thalassemia, and the regimen of HD-CTX led to a complete hematologic remission.</p>

Pure red cell aplasia with thymoma/T-cell large granular lymphocyte leukemia: two cases report and literatures review / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical and laboratory features of 2 cases of pure red cell aplasia (PRCA) with thymoma/T-cell large granular lymphocyte leukemia (T-LGLL), and to improve the recognition of the disease and the role of lymphocyte in its mechanism.</p><p><b>METHODS</b>Two cases of PRCA with thymoma/T-LGLL were reported and the related literatures were reviewed.</p><p><b>RESULTS</b>Case 1 was a 63-years old male with hemoglobin level of 54 g/L at admission. Case 2 was a 52-years old female with hemoglobin level of 79 g/L at admission. They were both diagnosed as PRCA with thymoma before admission to our hospital and had no benefit from their thymectomy. Further examinations in our hospital showed that CD3⁺CD4⁻CD8⁺CD57⁺ large granular lymphocytes amplified with clonal TCR rearrangement in their peripheral blood. The diagnosis of PRCA with thymoma/T-LGLL was clarified. Case 1 did not respond to any of the frontline therapies while case 2 responded completely to cyclosporine.</p><p><b>CONCLUSION</b>Both thymoma and T-LGLL could be the cause of secondary PRCA, lymphocyte proliferation may play critical role in the pathogenesis.</p>

Congenital dyserythropoietic anemia type II: a case report and literature review / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical and laboratory features of congenital dyserythropoietic anemia type II (CDA-II) in order to improve the recognition of the disease.</p><p><b>METHODS</b>A case of CDA-II was reported and the related literatures were reviewed.</p><p><b>RESULTS</b>The 32-years old female presented with moderate anemia, jaundice and hepatosplenomegaly from her childhood and was misdiagnosed as hereditary spherocytosis for a long time. There were no increased reticulocytes in the peripheral blood and her bone marrow showed erythroid hyperplasia with 43% of binucleated erythroblasts. Electron microscopy examination revealed stretches of double membrane lining the inner surface of the erythroblast cell membrane.</p><p><b>CONCLUSIONS</b>CDA-II is a rare congenital anemia characterized by ineffective erythropoiesis with unique laboratory features, and is relatively easy to be misdiagnosed. It is necessary to improve the awareness of CDA-II, and to set-up its responsible gene analysis, i.e., CDAN2 gene and SEC23B gene detection.</p>

Biomechanical properties study of aorta in β-aminopropionitrile-induced rat model / 中华外科杂志

<p><b>OBJECTIVE</b>To investigate thoracic aortic longitudinal elastic strength in β-aminopropionitrile (BAPN) treated rat model of aortic dissection (AD).</p><p><b>METHODS</b>Twenty-nine young rats (Sprague-Dawley) were divided into tow groups, control group (n = 12) and BAPN group (n = 17). Seventeen rats were treated with 0.25% BAPN mixed in feed for 6 weeks. All the rats were sacrificed in the end of experiment and aorta was harvested for biomechanical and pathological study. Longitudinal elastic strength and stress were detected and analyzed by material testing machine. Elasticity modulus as well as maximum stretching length, draw ratio, maximum load, maximum strength, and maximum extensibility was calculated according to the analysis with thickness and area of aortic media.</p><p><b>RESULTS</b>Nine BAPN-treated rats died of aortic dissecting aneurysm rupture during the experiment. The diameter of the aneurysms was (6.33 ± 1.17) mm and the length was (9 ± 5) mm. The maximum diameter significantly increased in BAPN-induced rats with AD (group B2) compared with without AD (group B1) and control group ((6.49 ± 1.20) mm vs. (1.45 ± 0.11), (1.25 ± 0.26); F = 165.257, P = 0.001 and 0.000, respectively), but there was no significance between group B1 and control group (P = 0.108). Thickness and area of aortic media in BAPN-induced rats significantly increased compared with control group (F = 27.277 and 27.153, P = 0.000 and 0.000, respectively), but there was no significance of area between group B1 and B2 (P = 0.540). Maximum stretching length, draw ratio, maximum load, maximum strength maximum extensibility and elasticity modulus were significantly decreased from group B2, group B1 to control group (P < 0.01, respectively).</p><p><b>CONCLUSIONS</b>This study built a successful model of AD. Biomechanical analysis and the decrease of maximum stretching length, draw ratio, maximum load, maximum strength maximum extensibility and elasticity modulus may explain the formation of AD partly.</p>